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Articole Multimedia Jurnalul de Chirurgie, Iaşi, 2008, Vol. 4, Nr. 4 [ISSN 1584 – 9341] THE ADRENOCORTICAL CARCINOMA G. Favia University of Padova Department of Science Surgery and Gastroenterology Conference presented at 27-th National Congress of Italian Society of Endocrine Surgery, Cagliary, Italy, October 2008 THE ADRENOCORTICAL CARCINOMA G. Favia University of Padova Department of Surgical Science and Gastroenterology Abstract: Adrenocortical carcinoma (ACC) is a rare tumor with a poor prognosis. Overall incidence is about 0.5-2 / milion inhabitants. ACC could be secreting (about 60% from all the cases) or non-secreting (diagnosed espacially as an incidentaloma during an ultrasound or CT-scan exam). The paper discuss the difficulties of positive diagnosis for ACC, from point of view of histological exam (Weiss criteria), biological markers (e.g. Ki67, p53), imagery techniques (CT-scan, IRM, scintigraphy). Treatment of the ACC is also challenging; radical surgery is possible in early stages. Large „en bloc” resections it is also recommended in locally advance stages. The place of adjuvant therapy and reoperations for recurrence is also discussed. The experience in ACC surgery of the Department of Surgery from Padova University is also presented. Conclusions: ACC carries a poor prognosis for patients commonly presenting with large, locally invasive tumors, and metastatic disease. A precocious diagnosis using imagery and histologic criteria, aggressive surgery and novel therapies may help to increase survival, which has remained unchanged over the last 20 years. KEY WORDS: ADRENOCORTICAL CARCINOMA, DIAGNOSIS, PRONOSTIC FACTORS, ADRENALECTOMY[*] [*]Conference presented at 27-th National Congress of Italian Society of Endocrine Surgery, Cagliary, Italy, October 2008 ADRENOCORTICAL CARCINOMA ADRENOCORTICAL CARCINOMA • Incidence: 0.5-2 / milion inhabitants /year • Preoperative Diagnosis - “Difficult” • Histological Diagnosis - “Difficult”: FOLLOW UP • Surgery is “Radical”(?): RECCURENCES REINTERVENTIONS • SECRETING (60%) •30% HYPERCORTISOLISM •22% VIRILITY •10% FEMININITY •3%HYPERALDOSTERONISM •35% 35% MIXTED SECRETIONS • NON-SECRETING (40%) • INCIDENTALOMA THE SURGERY OF ADRENOCORTICAL CARCINOMA NEW ISSUES What had been changed ? IMPROVEMENT of HISTOLOGICAL DIAGNOSIS IMPROVEMENT of RADIOLOGICAL DIAGNOSIS AGGRESSIVE SURGERY ADJUVANT TERAPY(?) 325
Articole Multimedia Jurnalul de Chirurgie, Iaşi, 2008, Vol. 4, Nr. 4 [ISSN 1584 – 9341] ADRENOCORTICAL CARCINOMA HISTOLOGICAL DIAGNOSIS WEISS CRITERIA 1. HIGH DEGREE OF NUCLEATION 2. >5 MITOSIS PER 50 HPF 3. ATIPICAL MITOSIS 4. CITOPLASMATIC EOSINAFILY (>75% cellule tumorali) 5. FIBROSIS AND TRABECOLARY STRIPS 6. NECROSIS 7. VENOUS INVASION 8. SINUSOIDAL INVASION 9. CAPSULAR INFILTRATION WEISS < 3 WEISS > 4 0 1 2 3 4 5 6 7 ADRENOCORTICAL CARCINOMA HISTOLOGICAL DIAGNOSIS WEISS CRITERIA HIGH DEGREE OF NUCLEATION >5 MITOSIS PER 50 HPF ATYPICAL MITOSIS CYTOPLASMATIC EOSINOFILIA FIBROSIS NECROSIS VENOUS INVASION CAPSULAR INFILTRATION Adenoma Carcinoma Weiss, 95 WEISS < 3 ADRENOCORTICAL CARCINOMA HISTOLOGICAL DIAGNOSIS WEISS > 4 0 1 2 3 4 5 6 7 ADRENOCORTICAL CARCINOMA HISTOLOGICAL DIAGNOSIS •Ki-67 ■p53p53 Adenoma Carcinoma ??? PROBLEMATIC CASES: Just the present or the appearance of recurrence or the systemic metastasis could confirm the diagnosis of MALIGNITY ‣Higher in Carcinoma (36%) lower in Adenoma (0%) ‣Correlation with Survival We need other MARKERS !!! Diagnosis criteria Prognosis factor Martins 2005 Stojadinovic 2003 ADRENOCORTICAL CARCINOMA HISTOLOGICAL DIAGNOSIS • Insulin like Growth Factor II (IGF 2) ADRENOCORTICAL CARCINOMA IMAGERY DIAGNOSIS Growth factor (cromosome 11 p15) ‣Higher in Carcinoma (90%) lower in Adenoma (0%) ‣Correlation with survival Diagnosis criteria Prognosis factor Sidhu 2003 General speaking, it is easy to establish the diagnosis of “adrenal tumor”and in some cases to establish the diagnosis of adrenal carcinoma (e.g. invasion, metastasis). 326
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